Behçet's disease diagnostic study of choice
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Cafer Zorkun, M.D., Ph.D. [2]
Overview
Currently, there is not a specific test to confirm the diagnosis of Behçet's disease. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks obtained by a thorough history of the patient's symptoms (outlined below). Behçet's disease is a diagnosis of exclusion, and other chronic inflammatory diseases should be evaluated for. The various inflammatory symptoms do not necessarily occur together, and they will vary in severity.
Diagnostic Criteria
There are three levels of certainty for diagnosis:
- International Study Group diagnostic guidelines (very strict for research purposes)
- Practical clinical diagnosis (generally agreed pattern but not as strict)
- 'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)
International Study Group diagnostic guidelines
Must have
- oral (aphthous) ulcers (any shape, size or number at least 3 times in any 12 months),
along with 2 out of the next 4 "hallmark" symptoms:
- genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men),
- skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids),
- eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous),
- pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick).
Practical clinical diagnosis
Must have
- mouth ulcers,
along with 1 of the 4 hallmark symptoms above and with 2 of the symptoms below:
- arthritis/arthralgia,
- nervous system symptoms,
- stomach and/or bowel inflammation,
- deep vein thrombosis,
- superficial thrombophlebitis,
- cardio-vascular problems of inflammatory origin,
- inflammatory problems in chest and lungs,
- problems with hearing and/or balance,
- extreme exhaustion,
- changes of personality, psychoses,
- any other members of the family with a diagnosis of Behcet disease.
'Suspected' or 'Possible' diagnosis
Usually assigned when someone does not have mouth ulcers, or has mouth ulcers but does not have 1 of the 4 hallmark symptoms but has other symptoms and signs of inflammation, and other causes for these have been ruled out.